Volume 11, Issue 2 (8-1997)                   Med J Islam Repub Iran 1997 | Back to browse issues page

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MOIN M, FARHOUDI A, MOIN A. PAPILLON-LEFE'VRE SYNDROME: NEUTROPHIL MOTILITY AND KILLING DEFECT IN A CHILD WITH RECURRENT SEVERE INFECTIONS. Med J Islam Repub Iran 1997; 11 (2) :149-152
URL: http://mjiri.iums.ac.ir/article-1-1122-en.html
PAPILLON-LEFE'VRE SYNDROME: NEUTROPHIL MOTILITY AND KILLING DEFECT IN A CHILD WITH RECURRENT SEVERE INFECTIONS
M MOIN , ABOLHASAN FARHOUDI , ATHAR MOIN
From the Department of Clinical Immunology and Allergy, Children's Medical Center, Tehran University of Medical Sciences, Tehran
Abstract:   (5243 Views)
A case of palmoplantar hyperkeratosis with periodontosis and a history of recurrent severe pyoderma, pneumonia and multiple liver abscesses is described in a 12 year old girl. The patient demonstrated neutrophil dysfunction characterized by decreased random migration and chemotaxis and defective bactericidal activity. The exact immunopathological mechanism for susceptibility to infections in Papillon-Lefe'vre syndrome patients still remains to be determined. However, the mode of clinical presentation, laboratory findings and response to retinoid treatment, all support the speculation of Papillon-Lefe'vre syndrome as a primary immunologic disease with a variable defect in neutrophil motility and bactericidal activity. The pattern of clinical presentations as skin and periodontal lesions alone or with susceptibility to infection in other sites will change accordingly.
Keywords: Keratosis, periodontosis, neutrophil, chemotaxis, chemiluminescense
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: case report | Subject: Immunology
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